World Journal for Pediatric & Congenital Heart Surgery

Circumflex aorta is described as a retroesophageal aortic arch, with opposite-sided descending aorta forming a true vascular ring with ligamentum arteriosum. We report two cases of right-sided circumflex aorta with varied clinical presentation. Computed tomography diagnosed this vascular ring anomaly. The patients were managed with the aortic uncrossing procedure as a primary surgical strategy. Preoperative diagnosis and thorough planning were essential for the successful outcome. The patients did not have any residual symptoms of tracheal and esophageal compression on follow up...

BACKGROUND: This study examined preoperative, intraoperative, and postoperative data to identify factors that are associated with prolonged mechanical ventilation (PMV) and prolonged intensive care unit length of stay (ICU LOS) in tetralogy of Fallot (TOF) patients undergoing repair surgery. METHODS: A retrospective study was carried out after approval from the institutional review board. All patients (age 0-52 years) who underwent TOF repair from January 2016 to September 2022 were included...

We describe the perventricular device closure of a large mid-muscular ventricular septal defect (VSD) in a 2.9 kg infant born with hypoplastic aortic arch and VSD using an Occlutech perimembranous occluder. In this case, the anatomy required a short low-profile device and hence a perimembranous occluder was used. To our knowledge, this is the first described use of this device for hybrid closure of a muscular VSD and the application of this technique in a patient <3 kg.

The recent special issue of the World Journal for Pediatric and Congenital Heart Surgery devoted to hypoplastic left heart syndrome, and its related anomalies, contained significant information of great clinical relevance. Very little attention, however, was devoted to the integrity of ventricular septum as providing a criterion to distinguish between the phenotypes to be included within the syndrome, as opposed to the related anomalies. In this commentary, we summarize the evidence in support of the notion that the phenotypes to be included within the syndrome can be interpreted on the basis of an acquired disease of fetal life...

Surgical repair of right aortic arch and aberrant left subclavian artery has traditionally involved ligamentum division. Such patients can have stenosis at the origin of the aberrant subclavian artery either at the time of presentation or later. The more recently popularized repair involving resection of Kommerell diverticulum with transfer of the subclavian artery to the left carotid artery allows resection of the stenotic segment and serves as an effective treatment for subclavian stenosis as well. We present three cases of early repair of this arch anomaly with associated subclavian stenosis repaired successfully in that manner...

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BACKGROUND: Controversy regarding the optimal pulmonary valve substitute remains, with no approved surgical valve for pulmonary valve replacement (PVR). Furthermore, unfavorable anatomy often precludes transcatheter PVR in patients with congenital heart disease. We therefore sought to evaluate the feasibility of the Edwards Inspiris pericardial aortic bioprosthesis in the pulmonary position in pediatric and adult patients requiring PVR. METHODS: Data from consecutive patients who underwent PVR from February 2019 to February 2021 at our institution were retrospectively reviewed...

Percutaneous ductal device closure in neonates is gaining popularity. Cardiac perforation is a rare but catastrophic complication that can occur during this procedure. Surgical options to salvage this situation are limited in extremely low-weight babies. In this report, we describe one such case managed successfully and offer some suggestions to achieve a successful outcome.

Atrioventricular septal defect (AVSD) with shunting restricted to the ventricular level is a rare form of AVSD. To our knowledge, this shunting pattern has not been reported in AVSD with tetralogy of Fallot. We report a child with this unusual combination who underwent a successful single-stage repair at two years of age.

Tricuspid valve (TV) dysplasia is a rare congenital defect that, in severe cases, can cause heart failure necessitating surgical correction. TV replacement options are limited in neonates since few commercial valves are available and the rates of failure and postoperative mortality are high. The authors report a neonate with TV dysplasia who underwent replacement with a hand-constructed tube valve using small intestinal submucosal extracellular matrix, which functioned well for >5 years.

Aortic arch obstruction is often present with complex concomitant congenital heart defects (CHDs). The use of nonvalved femoral vein homograft (FVH) to reconstruct the aortic arch has distinct surgical advantages, including simplified reconstruction. We present an intraoperative video of a Yasui procedure utilizing FVH for aortic reconstruction in a 12-day-old (2.2 kg) neonate with right ventricular outflow tract obstruction, malalignment ventricular septal defect, aortic valve atresia, aortic arch hypoplasia, atrial septal defect, and ductal dependent systemic circulation...

A previously healthy nine-year-old boy with anomalous aortic origin of the left coronary artery (AAOLCA) with high-risk anatomy demonstrated negative stress on magnetic resonance imaging. Invasive cardiac catheterization for intracoronary flow measurement was performed and demonstrated compromised coronary flow during pharmacologic stress and significant stenosis on angiography. The patient underwent surgical intervention with normalization of coronary flow upon postoperative evaluation. Invasive intracoronary flow determination with angiography under provocative stress is emerging as a critical data point for risk stratification and management decision-making in high-risk AAOLCA patients with negative noninvasive perfusion studies...

Double-chambered left ventricle (DCLV) is a rare congenital cardiac lesion. It is usually an incidental finding in children and young adults. Diagnosis during fetal life is exceedingly rare. We report a case of DCLV diagnosed in the prenatal period associated with a ventricular septal defect (VSD). Transthoracic echocardiographic and magnetic resonance imaging confirmed the prenatal findings. At seven-month follow-up, the baby was asymptomatic. Transthoracic echocardiographic check-up revealed spontaneous closure of the VSD and stable aspect of the main left ventricle and the accessory chamber...

Ganglioneuroma is a benign tumor requiring subtotal resection as a primary mode of treatment. There are several surgical approaches. A giant ganglioneuroma of the chest cavity may be approached via a clamshell thoracotomy. This manuscript presents a case of giant ganglioneuroma resected en bloc via clamshell thoracotomy in a seven-year-old child.

Sinus of Valsalva aneurysm (SVA) is an abnormal dilatation of the aortic root located between the aortic valve annulus and the sinotubular junction and is rare in the pediatric population. This case report describes a unique case of a 16-year-old adolescent patient admitted with progressive heart failure symptoms and diagnosed with a ruptured noncoronary SVA. He underwent surgical repair of the SVA with autologous pericardial patches and had an uncomplicated postoperative course. A genetic workup revealed an underlying 22q11...

BACKGROUND: The use of extracorporeal membrane oxygenation (ECMO) in the postoperative cardiac critical care setting is evolving. Anticoagulation monitoring is among the most challenging aspects of pediatrics. However, there is no consensus on the optimal dosing and monitoring of unfractionated heparin in this setting. To address this, we developed an anti-Xa assay-based protocol derived from the best available clinical and anecdotal evidence of ECMO use and assessed its effectiveness in achieving the anti-Xa assay therapeutic target...

Background: While progress has been made to decrease mortality in children under age five, there continues to be a need for improvement in the treatment of children with congenital heart disease. Many of these patients require surgical correction and live in areas without the expertise of surgical teams. Research has shown that appropriate training is critical to ensure the best clinical outcomes. The Ethiopian government has identified the need for increased training of health care professionals as a method to improve hospital outcomes...

This consensus document for the performance of cardiovascular computed tomography (CCT) to guide intervention in the right ventricular outflow tract (RVOT) in patients with congenital heart disease (CHD) was developed collaboratively by pediatric and adult interventionalists, surgeons, and cardiac imagers with expertise specific to this patient subset. The document summarizes definitions of RVOT dysfunction as assessed by multimodality imaging techniques and reviews existing consensus statements and guideline documents pertaining to indications for intervention...

We report a case of a one-year-old boy with tetralogy of Fallot, who was preoperatively diagnosed to have an associated systemic venous anomaly. Computed tomography confirmed the absent superior vena cava, and the case was managed with an appropriate cannulation strategy. Preoperative diagnosis and thorough planning of this rather benign anomaly were imperative for the successful outcome of this case. Clinical and surgical implications of this anomaly are discussed in this report.

BACKGROUND: We reviewed the outcomes of 82 consecutive pediatric patients (less than 18 years of age) supported with the Berlin Heart ventricular assist device (VAD), comparing those with congenital heart disease (CHD; n  =  44) with those with acquired heart disease (AHD; n  =  37). METHODS: The primary outcome was mortality after VAD insertion. Kaplan-Meier methods and log-rank tests were used to assess group differences in long-term survival...